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Fournier's Gangrene: Not Your Garden Variety Genital Infection




CASE

A middle-aged male patient with a past medical history of diabetes mellitus (uncontrolled), HTN, and necrotizing fasciitis of the buttocks and perineum presents to the Emergency Department at night with one week of suprapubic pain and rash as well as penile pain and discharge. History and physical exam reveal the patient was hospitalized earlier this year (April 2022) for necrotizing fasciitis requiring surgical intervention and inpatient antibiotic therapy. Since this time, he had not been adhering to the hygiene of his groin, he had been consuming a high-sugar diet, and he had been poorly compliant with medications. The patient denied fever, chills, nausea, or vomiting. He endorsed tenderness over the affected area as well as urinary incontinence. The patient’s physical exam showed erythema and warmth over the left suprapubic region, penile discharge and erythema, and scrotal skin irritation due to urinary incontinence. The patient’s vital signs were within normal limits, and he had a normal white blood cell count on CBC.


Clinical Question: What alarm signs should we look for to manage this emergent diagnosis?


SUMMARY OF EVIDENCE

What is Fournier’s Gangrene (FG)?

  • FG, a relatively rare form of necrotizing fasciitis, is a rapidly progressive disease that affects the deep and superficial tissues of the perineal, anal, scrotal, and genital regions (1).

  • The disease has a higher incidence in males, and risk factors for development include diabetes mellitus, HIV, alcoholism, recent surgery, vascular disease, drug use, and other immune-compromised states (2,3).

  • Patients present initially with systemic symptoms, such as fever and chills, as well as local symptoms, such as cellulitis and a swollen, erythematous, and tender penis, scrotum, inguinal area, or perineum (4). Genitourinary symptoms include dysuria and obstructed voiding. These symptoms rapidly progress to crepitus and dark purple or black skin discoloration (4).

What is Included in the Differential Diagnosis?

· Scrotal Cellulitis, Epididymitis, Scrotal Abscess, Tinea Cruris, Scrotal Wall Hematoma, Testicular Trauma (5).

How Do We Diagnose Fournier’s Gangrene?

  • FG is a clinical diagnosis. There are no specific laboratory or imaging studies that can specifically rule in or out the disease (3).

  • Early-stage FG is a difficult diagnosis to make and is misdiagnosed in up to three-quarters of cases, contributing to higher mortality as the infection progresses (3).

  • With FG being a clinical diagnosis, Talwar (2010) outlined a natural history of disease course (6):

    • Prodromal symptoms of fever and lethargy may be present for two to seven days (6)

    • Intense genital pain and tenderness are usually associated with overlying skin edema (6)

    • Increasing genital pain and tenderness with progressive erythema of the overlying skin (6)

    • Dusky appearance of the overlying skin; subcutaneous crepitation (6)

    • Obvious gangrene of a portion of the genitalia; purulent drainage from the wounds (6)

  • Risk calculators to prognosticate and predict the necessity of intervention for FG include the Fournier’s Gangrene Severity Index (FGSI) and the Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score (3). The LRINEC score is a useful clinical determinant in the diagnosis and surgical treatment of patients with necrotizing fasciitis, with a statistically positive correlation between the LRINEC score and an accurate diagnosis of necrotizing fasciitis. (Bechar).

  • Conventional radiography is often used to rule out necrotizing soft tissue infection (NSTI) based on absent soft tissue emphysema. However, emphysema is most specific to clostridial infection (3)

  • CT imaging can assist with diagnosis and planning for surgical management. CT has high sensitivity (88.5%) and specificity (93.3) for NSTI diagnosis. (3).

  • Point of care ultrasound (POCUS) has shown high sensitivity (88%) and specificity (93%) for subcutaneous thickening, air, and fascial fluid (3). The presence of intrascrotal gas is a pathognomonic sign of NSTI (8)

RECOMMENDATIONS

What are the Differentiating Alarm Signs?

With the difficulty of subjectivity regarding the diagnosis of FG, what alarm signs are highly indicative of diagnosis and should alert immediate intervention? Physicians with suspicion of FG should elevate it on their differential diagnosis if they find:

  • Patients with current or history of diabetes mellitus, HIV, alcoholism, recent surgery, vascular disease, drug use, local trauma, instrumentation, and perirectal or perianal infections (2,3,9)

  • Marked systemic toxicity out of proportion to local findings (9)

  • Palpable crepitus underneath the skin, which is sufficient to make the diagnosis (9)

  • Dark purple or black discoloration of the skin (9)

What is the Management and Disposition?

Management of FG includes surgical debridement, broad-spectrum antibiotics, and resuscitation with intravenous fluids and vasopressors (10). Antibiotic therapy includes coverage of streptococcal, staphylococcal, and gram-negative bacteria; coliforms; Pseudomonas; Bacteroides; and Clostridium (3). Disposition of the patient includes prompt surgical consultation and intervention with subsequent intensive care unit management to administer antibiotic therapy and post-operative management (3).


REFERENCES

1. Stephen L, Rad J, Foreman J. Fournier gangrene - statpearls - NCBI bookshelf. Fournier Gangrene. https://www.ncbi.nlm.nih.gov/books/NBK549821/. Published June 17, 2022. Accessed October 8, 2022.

2. Singh A;Ahmed K;Aydin A;Khan MS;Dasgupta P; Fournier's gangrene. A clinical review. Archivio italiano di urologia, andrologia : organo ufficiale [di] Societa italiana di ecografia urologica e nefrologica. https://pubmed.ncbi.nlm.nih.gov/27711086/. Published October 5, 2016. Accessed October 8, 2022.

3. Bornstein K, Ramzy M, Cabrera J, Montrief T, Long B. Fournier gangrene in the emergency department: Diagnostic Dilemmas, treatments and current perspectives. Open access emergency medicine : OAEM. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7665443/. Published November 9, 2020. Accessed October 9, 2022.

4. Hofer MD. Urologic emergencies. Medical Clinics of North America. https://www.sciencedirect.com/science/article/pii/S0025712517301670. Published December 20, 2017. Accessed October 8, 2022.

5. Holtz M, Swartz J, Snyder A, Cunningham R, Donaldson R, Lu K. Fournier Gangrene. WikEM. https://wikem.org/wiki/Fournier_gangrene. Published February 9, 2021. Accessed October 9, 2022.

6. Talwar A, Puri N, Singh M. Fournier’s Gangrene of the penis: a rare entity. J Cutan Aesthet Surg. 2010;3(1):41–44. doi: 10.4103/0974-2077.63394

7. Bechar J, Sepehripour S, Hardwicke J, Filobbos G. Laboratory risk indicator for necrotising fasciitis (LRINEC) score for the assessment of early necrotising fasciitis: A systematic review of the literature. Annals of the Royal College of Surgeons of England. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5449710/. Published May 2017. Accessed October 9, 2022.

8. Di Serafino M, Gullotto C, Gregorini C, Nocentini C. A clinical case of Fournier’s gangrene: imaging ultrasound. J Ultrasound. 2014;17(4):303–306. doi: 10.1007/s40477-014-0106-5

9. Manjunath AS, Hofer MD. Urologic emergencies. Medical Clinics of North America. https://www.sciencedirect.com/science/article/pii/S0025712517301670. Published December 20, 2017. Accessed October 9, 2022.

10. Hakkarainen TW, Kopari NM, Pham TN, Evans HL. Necrotizing soft tissue infections: review and current concepts in treatment, systems of care, and outcomes. Curr Probl Surg. 2014;51(8):344–362. doi: 10.1067/j.cpsurg.2014.06.001

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